1.Rare Case: Empty Sella Syndrome, Growth Hormone Deficiency, and Hashimoto’s Thyroiditis in Thalassemia Spectrum
Athari Fadhila Namanda Putri ; Eva Decroli ; Dinda Aprilia ; Alexander Kam ; Yanne Pradwi Efendi
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):95-
Introduction:
Thalassemia is a hemoglobin synthesis disorder
associated with chronic anemia and transfusion-related
iron overload, which predisposes patients to multiple
endocrine complications. Iron deposition in the pituitary
and gonads may result in growth hormone deficiency
(GHD) and hypogonadism. Empty sella syndrome (ESS),
characterized by herniation of the subarachnoid space into
the sella turcica, may also contribute to hypopituitarism.
The coexistence of thalassemia-related endocrinopathies,
ESS, and autoimmune thyroid disease is rare and presents
significant diagnostic complexity.
Case:
A 27-year-old female with transfusion-dependent
thalassemia on deferiprone presented with secondary
amenorrhea and short stature. Her height was 145 cm
(below the target range of 140.5–157.5 cm), body mass
index 17.3 kg/m², and Tanner stage M3P1.
Laboratory evaluation revealed elevated thyroidstimulating hormone (10.92 mIU/L) with low-normal
free thyroxine 4 (11.4 pmol/L), consistent with primary
hypothyroidism. Thyroid ultrasound demonstrated features of chronic thyroiditis, and anti-thyroid peroxidase
antibodies (8.18 IU/mL) supported a diagnosis of
Hashimoto’s thyroiditis. Insulin-like growth factor-1
was markedly reduced (68 ng/mL), indicating GHD.
Morning cortisol was within normal range (14.5 µg/dL).
Gonadotropins were inappropriately low-normal (folliclestimulating hormone 7.42 mIU/mL, luteinizing hormone
11.41 mIU/mL) with low estradiol (26.49 pg/mL), suggesting
hypogonadotropic hypogonadism.
Skeletal survey showed thalassemia-related bone changes,
including trabecular coarsening and metaphyseal widening,
with a predicted adult height of 142.7 cm. Pituitary magnetic
resonance imaging revealed an empty sella.
Conclusion
Thalassemia must be recognized not only as a primary
hematologic condition but also as a complex multisystem
disorder with profound endocrine implications.
Furthermore, the co-occurrence of these conditions with
rare manifestations such as ESS and Hashimoto’s thyroiditis
underscores the diagnostic complexity faced by clinicians.
Therefore, early detection and rigorous, regular endocrine
screening are essential to optimize management strategies
and improve the long-term quality of life for patients with
thalassemia.
Empty Sella Syndrome
;
Growth Hormone
;
Thalassemia
;
Thyroiditis
2.Unusual presentation of Sheehan's syndrome with severe hyponatremia and recurrent symptomatic hypoglycemia: A case report.
Siti Adewiah ; Abdullah ; Maimun Syukri ; Hendra Zufry ; Krishna Wardhana Sucipto
Journal of the ASEAN Federation of Endocrine Societies 2016;31(2):166-170
Sheehan's syndrome (SS) is postpartum hypopituitarism caused by necrosis of the pituitary gland. The onset in most cases is several months or even years after the inciting delivery, so it is often unrecognized and not adequately treated. Because SS often evolves slowly, it is usually diagnosed late. We report a 47-year old woman with loss of consciousness. Fourteen years ago, she had postpartum hemorrhage with subsequent amenorrhea and failure to lactate. Laboratory investigation showed low blood sugar and serum sodium levels, amid normal cortisol and thyroid function tests. Magnetic resonance imaging (MRI) of the pituitary revealed an empty sella consistent with SS. The presentation of hypoglycemia and hyponatremia are less known complications of Sheehan's syndrome with only a few documented in case reports.
Human ; Female ; Middle Aged ; Amenorrhea ; Empty Sella Syndrome ; Hydrocortisone ; Hypoglycemia ; Hyponatremia ; Hypopituitarism ; Lactic Acid ; Magnetic Resonance Imaging ; Pituitary Diseases ; Pituitary Gland ; Postpartum Hemorrhage ; Thyroid Function Tests ; Unconsciousness ; Growth Hormone ; Hormones
4.Polyglandular Autoimmune Syndrome Type 2 Complicated by Multiple Organ Failure, Empty Sella Syndrome and Aplastic Anemia.
Hyun Je KIM ; Seung Il BAE ; Young Hoon HONG
Journal of Rheumatic Diseases 2015;22(2):111-117
Polyglandular autoimmune syndrome (PAS) is a group of syndromes comprised of glandular and extra-glandular disorders characterized by autoimmunity. A 57-year-old woman presented with acute progressive dyspnea and generalized weakness for several months. The patient was assessed to have acute congestive heart failure with cardiomyopathy, chronic renal failure with hyporeninemic hypoaldosteronism, and pancytopenia in addition to primary hypothyroidism and adrenal insufficiency. With the diagnosis of PAS type 2 complicated by multiple organ failure (MOF), medium-dose prednisolone (30 mg/d) was introduced primarily to control the activity of autoimmunity, which triggered MOF over the adrenal insufficiency. Levothyroxine (25 microg/d) was followed for replacement of the thyroid hormone deficiency. However, the symptoms and signs fluctuated, depending on the dosage of prednisolone, and progressively worsened by empty sella syndrome and aplastic anemia. Here, we report on a case of PAS type 2 with MOF and atypical complications, and suggest that recognition, assessment, and control of PAS as a systemic autoimmune disease may be essential.
Adrenal Insufficiency
;
Anemia, Aplastic*
;
Autoimmune Diseases
;
Autoimmunity
;
Cardiomyopathies
;
Diagnosis
;
Dyspnea
;
Empty Sella Syndrome*
;
Female
;
Heart Failure
;
Humans
;
Hypoaldosteronism
;
Hypothyroidism
;
Kidney Failure, Chronic
;
Middle Aged
;
Multiple Organ Failure*
;
Pancytopenia
;
Prednisolone
;
Thyroid Gland
;
Thyroxine
5.Related factors analysis of spontaneous cerebrospinal fluid leak recurrence after endoscope operation.
Huanxin YU ; Haiyan LI ; Gang LIU
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2015;29(21):1847-1849
OBJECTIVE:
To Summarize and analyze the clinical characteristics and treatment of patients with spontaneous cerebrospinal fluid rhinorrhea, and to explore the related factors of recurrence.
METHOD:
Retrospective- ly analyze the clinical data of 58 patients of spontaneous cerebrospinal fluid rhinorrhea from July 2002 to July 2012, combined with its clinical characteristics, and statistically analyze the related recurrence factors.
RESULT:
Fifty-eight cases were accepted the nasal endoscopic repairment of cerebrospinal fluid rhinorrheak, follow-up 3 years, 20 cases (34.5%) recurred, 1 case recurred in half a year after operation. In the first year, there were 10 cases recurred. In the second year there were 9 cases recurred, and 1 case recurred in the third year. Through multiariable analysis it was found that higher BMI, empty sella and skull base bone defect were the independent risk factors influencing the recurrence of spontaneous cerebrospinal fluid rhinorrhea (P < 0.05).
CONCLUSION
The recurrence rate of spontaneous cerebrospinal fluid rhinorrhea is high, needing long-term follow-up. Higher BMI, empty sella, skull base bone defect are independent risk factors of recurrence of the disease, and the treatment should be individualized.
Cerebrospinal Fluid Rhinorrhea
;
pathology
;
surgery
;
Empty Sella Syndrome
;
pathology
;
Endoscopy
;
adverse effects
;
Humans
;
Nose
;
Recurrence
;
Retrospective Studies
;
Risk Factors
;
Skull Base
;
pathology
6.A Case of Primary Empty Sella Syndrome with Central Diabetes Insipidus.
Ji Soo HAN ; Hong Woo CHUN ; Hye Min JO ; Jung Hyun KIM ; Kyu Jin KIM ; Chan Hee JUNG ; Ji Oh MOK ; Chul Hee KIM ; Sung Koo KANG ; Bo Yeon KIM
Soonchunhyang Medical Science 2014;20(2):172-175
Primary empty sella syndrome is a phenomenon caused by cerebrospinal fluid filling resulting from the herniation of the subarachnoid space within the sella. The pituitary function of primary empty sella syndrome is usually normal. But sometimes this syndrome causes some degree of pituitary dysfunction associated with hypersecretion or deficiency of pituitary hormone. Central diabetes insipidus with primary empty sella syndrome is rarely reported. Furthermore, most of those cases are accompanied by other pituitary dysfunction. We report here on a 35-year-old female who suffered from polyuria, polydipsia since childhood. She was diagnosed with central diabetes insipidus with primary empty sella syndrome. She had no anterior pituitary dysfunction except mild hyperprolactinemia.
Adult
;
Cerebrospinal Fluid
;
Diabetes Insipidus, Neurogenic*
;
Empty Sella Syndrome*
;
Female
;
Humans
;
Hyperprolactinemia
;
Polydipsia
;
Polyuria
;
Subarachnoid Space
7.A Case with Empty Sella Syndrome Combined with Multiple Anterior Pituitary Hormone Deficiencies Presenting as Hypoglycemic Coma.
Soo Kyoung KIM ; Sung Su KIM ; Bo Ra KIM ; Jung Hwa JUNG ; Jae Hoon JUNG ; Jong Ryeal HAHM
Kosin Medical Journal 2014;29(2):151-155
A 55-year-old male was admitted to emergency department with a hypoglycemic shock of unknown origin. He was presented with tonic seizure activity after admission. Initial diagnostic procedure could exclude diabetes mellitus, drug side effects, and exogenous insulin application. Detailed evaluation of the patient's history revealed that the patient had experienced repeated hypoglycemic episodes for 2 years. He was diagnosed with hypothyroidism six years ago. Initial laboratory investigations revealed hypoglycemia, hyponatremia, and low plasma cortisol level (0.18 microg/dL). Sellar magnetic resonance imaging showed empty sella. Replacement therapy with hydrocortisone resulted in the improvement of clinical symptoms. Combined pituitary stimulation test with exception of hypoglycemia induced growth hormone and cortisol stimulation test was performed. The response of thyroid stimulating hormone, prolactin, follicle-stimulating hormone, and luteinizing hormone was normal. We report the case of empty sella syndrome associated with hypoglycemic shock due to with multiple anterior pituitary hormone deficiencies.
Coma*
;
Diabetes Mellitus
;
Emergency Service, Hospital
;
Empty Sella Syndrome*
;
Follicle Stimulating Hormone
;
Growth Hormone
;
Humans
;
Hydrocortisone
;
Hypoglycemia
;
Hyponatremia
;
Hypothyroidism
;
Insulin
;
Luteinizing Hormone
;
Magnetic Resonance Imaging
;
Male
;
Middle Aged
;
Plasma
;
Prolactin
;
Seizures
;
Shock
;
Thyrotropin
8.The characteristics and treatment of empty sella combined cerebrospinal fluid leakage of nasal.
Xiang ZHAI ; Jinling ZHANG ; Gang LIU
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2012;26(23):1063-1066
OBJECTIVE:
To study the feature and treatment method of patients with empty sella merger cerebro-spinal fluid leakage of nasal.
METHOD:
There were 8 cases with empty sella merger cerebrospinal fluid leakage of nasal, 2 cases were accepted the repairing surgery of cerebrospinal fluid leakage one time, 4 cases were accepted the repairing surgery of cerebrospinal fluid leakage used endoscope 2 times, 1 case was accepted repairing surgery of cerebrospinal fluid leakage used endoscope merge craniotomy and ventricle celiac bypass, 1 case recurrences after repairing surgery of cerebrospinal fluid was recurred after conservative treatment. Some postoperative were stayed in bed for three weeks and lumbar drainage for 1 week.
RESULT:
One case of cerebral hemorrhage after surgery was cured with craniotomy, followed for 2 years without recurrence. One case was recurred after conservative treatment. Two cases recurrences after surgery 3 years ago were accepted surgery again followed by one year without recurrence. One case who recurrence 1 year later was accepted repairing surgery of cerebrospinal fluid leakage used endoscope merge craniotomy and ventricle celiac bypass followed six months without recurrence. One cash after once surgery was followed half a year without recurrence. One case with recurrence 5 years later was accepted repairing surgery again.
CONCLUSION
The patient with empty sella combined cerebrospinal fluid leakage of nasal was rare, the main method was endoscopic sinus surgery treatment, but it recurred usually. The patients with repeatedly recurrence can be considered to accepted the surgery of ventricle celiac bypass. It required long-term postoperative follow-up and review.
Adult
;
Cerebrospinal Fluid Rhinorrhea
;
complications
;
diagnosis
;
surgery
;
Empty Sella Syndrome
;
complications
;
diagnosis
;
surgery
;
Endoscopy
;
Female
;
Humans
;
Male
;
Middle Aged
;
Nose
;
surgery
9.A Case of Endoscopic Repair of Cerebrospinal Fluid Rhinorrhea Associated with Primary Empty Sella Syndrome.
Jun Hyuk CHI ; Seok Won PARK ; Min Seok HAN ; Eun Kyeong LEE
Korean Journal of Otolaryngology - Head and Neck Surgery 2011;54(10):727-730
The empty sella syndrome is defined as the herniation of a subarachnoid space within the sella, with a flat-pressed pituitary gland accompanying hormonal problems or cerebrospinal fluid (CSF) rhinorrhea. CSF rhinorrhea is rare in the primary empty sella syndrome. We report a case of primary empty sella syndrome with CSF rhinorrhea in a 57 year-old woman. The CSF rhinorrhea was treated successfully by endoscopic transnasal transsphenoidal approach.
Cerebrospinal Fluid Rhinorrhea
;
Empty Sella Syndrome
;
Female
;
Humans
;
Pituitary Gland
;
Subarachnoid Space
10.Morphometric Study of the Korean Adult Pituitary Glands and the Diaphragma Sellae.
Kyo Sung JU ; Hack Gun BAE ; Hyung Ki PARK ; Jae Chil CHANG ; Soon Kwan CHOI ; Ki Bum SIM
Journal of Korean Neurosurgical Society 2010;47(1):42-47
OBJECTIVE: To investigate the morphometric characteristics of the pituitary gland and diaphragma sellae in Korean adults. METHODS: Using the 33 formaline fixed adult cadavers (23 male, 10 female), the measurements were taken at the diaphragma sellae and pituitary gland. The authors investigated the relationship between dura and structures surrounding pituitary gland, morphometric aspects of pituitary gland and stalk, and morphometric aspect of central opening of diaphragma sellae. RESULTS: The boundary between the lateral surface of pituitary gland and the medial wall of cavernous sinus was formed by the thin dural layer and pituitary capsule. The pituitary capsule adherent tightly to the pituitary gland was observed to continue from the diaphragma sellae. Mean width, length, and height of the pituitary gland were 14.3 +/- 2.1, 7.9 +/- 1.3, and 6.0 +/- 0.9 mm in anterior lobes, and 8.7 +/- 1.7, 2.9 +/- 1.1, and 5.8 +/- 1.0 mm in posterior lobes, respectively. Although all dimensions of anterior lobe in female were slightly larger than those in male, statistical significance was noted in only longitudinal dimension. The ratio of posterior lobe to the whole length of pituitary gland was about 27%. The mean thickness of pituitary stalk was 2 mm. The diaphragmal opening was 5 mm or more in 26 (78.8%) of 33 specimen. The opening was round in 60.6% of the specimen, and elliptical oriented in an anterior-posterior or transverse direction in 39.4%. CONCLUSION: These results provide the safe anatomical knowledge during the transsphenoidal surgery and may be helpful to access the possibility of the development of empty sella syndrome.
Adult
;
Cadaver
;
Cavernous Sinus
;
Diaphragm
;
Empty Sella Syndrome
;
Female
;
Formaldehyde
;
Humans
;
Male
;
Pituitary Gland


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